DIPNECH the unknown lung disease
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)
When people hear the words lung disease they think of COPD or black lung but there is another lung disease called DIPNECH. DIPNECH stands for Diffuse Idiopathic Pulmonary Neuroendocrine cell Hyperplasia. DIPNECH causes Neuroendocrine cells which are usually found in the intestines to invade the lungs thus causing slow growing Carcinoid tumors to form in the bronchioles. However these tumors differ from their counterparts since they are benign in nature.
Common symptoms are coughing, shortness of breath and sometimes wheezing. This disease mainly presents itself in non smoking middle aged women who have been previously diagnosed with Asthma. These women seek extra help when they realize regular asthma treatments are not making them feel better.
The tests used to confirm this disease are a Cat Scan, pulmonary function test and a lung biopsy. After it is a confirmed case of DIPNECH the proper treatment course can be implemented. However as of right now there aren't many treatments that are helpful for patients with DIPNECH. It is recommended that all patients diagnosed with DIPNECH be vaccinated for influenza, pneumonia and whooping cough since these diseases would hinder breathing even more than it already is.
Unfortunately basic inhalers like albuterol will not give patients any relief since their bronchiole obstruction can't be reversed like it can with asthma. Some patients do get relief form steroids whether they are in pill form like prednisone or the inhaled form like Spiriva but it is only temporary and certainly no cure.
There is another medicine that is called Sandostatin (the generic name is octreotide) and it is used in patients with intestinal Carcinoid tumors but it can also help those with DIPNECH. It is believed that the octreotide inhibits the pulmonary neuroendocrine cells (PNCs) enough to diminish the cough therefore making patients feel a little better.
It is imperative that all patients have their oxygen levels checked while both awake and sleeping since the PNC can cause more inflammation when they sense low oxygen in the blood stream. Because of low oxygen in the blood stream many patients require the use of oxygen 24/7 or at least a BIPAP machine while sleeping. The BIPAP machine "does two things - it helps push air into the lungs and helps hold the lungs open to allow more oxygen to enter the lungs. Each time the patient breathes, the BIPAP machine assists the patient by applying air pressure to the lungs while the patient is breathing out (exhaling or expiration) in order to hold open the air sacs in the lungs".(http://www.icu-usa.com/tour/equipment/bipap.htm)
When imaging shows Carcinoid tumors the best course of action is surgery. The purpose of surgery is to remove the infected portion of the lung so the healthy lung tissue can expand better thus improving the patients overall pulmonary functions. Lastly a lung transplant should be considered for those patients whose disease has progressed into severe airway obstruction and they have lost their ability to breathe on their own.
All information on DIPNECH can be found at (http://dipnech.com/DIPNECH_Patient_information.html)
With the medical explanations out of the way I can now explain how and why I know about this disease. I had never heard of DIPNECH until my mom was diagnosed with it two years ago when she finally went to National Jewish hospital here in Colorado. She started having asthma symptoms about thirty five years ago and was put on albuterol for treatment. She was doing quite well with a few colds etc here and there until 5 years ago when she was having a hard time catching her breath so she went her primary doctor and he put her on oxygen. However he never sent her to a pulmonologist partially because she didn't have insurance and because she didn't want to go to one. I finally pushed for her to go to National Jewish Hospital hospital 3 years later when she needed oxygen all the time but by then the disease had progressed to far and she was never able to go without it ever again. The pulmonologist at National Jewish said that she had lost about 40% of her breathing capacity and was very ill. He did put her on some steroid treatments but it only helped a little. With an actual pulmonologist following her and the treatments she was able to at least maintain what she had and not get any worse until she had to have surgery. My dad and I had to take her to the Emergency Room because she was having tremendous amount of stomach pain and that is when we learned she had a bowel obstruction and needed surgery. The surgery went well but her breathing got dramatically worse and she had to be put on a ventilator. The doctors tried to take her off the ventilator numerous times without any luck due to fact that her Carbon Dioxide levels would get to high making her very ill. It finally got to the point where she needed the ventilator all the time and became bedridden. After 5 months in the hospital her body couldn't handle the stress anymore and she had a massive heart attack which left her in a vegetative state. My Dad and I had to make one of the hardest decisions of our lives and we chose to turn off the life support because we knew my mom would never want to be that way. She died at the age 66 years old on May 21, 2010 at 9:45 pm leaving behind a daughter, husband and 3 grandchildren. However she touched so many lives that many of my friends referred to her as Mom and their children called her Granny.